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Wednesday, November 30, 2011

I have been avoiding this blog not because I am too busy but because I have been overwhelmed with what has been happening. Spencer continues to struggle. Struggle in ways I have never seen him struggle before and it's been scary to watch.

I am not even sure I can describe what is happening to him. At times, it's like watching someone have a stroke or blowing up a balloon then letting it zip at a furious speed until deflated within seconds. This is literally happening to Spencer. One minute he seems okay, not great but okay and without warning, he is completely deflated, no energy whatsoever. I don't mean he is just tired but can barely move his limbs, lift his head. It can last a few hours. Tuesday, it was scary. So scary even Sam and Shelbie were in shock to watch him. His eyes seem to glaze over and it's like he's gone, a switch of light flipped off.

I have no idea what is happening. I have emailed several of our doctors but have not received a reply yet. He has also developed a tremor in his hands, at times he can't even hold a pencil. Tonight, at dinner he said, "Mom, something bad is happening to me. Something is really wrong. I'm a little scared." He continued to tell me that the night before last, he woke up and couldn't feel his legs. He kept trying to move them but he couldn't move them, again, he was overcome with weakness.

I have heard from other mito moms and this sounds similar to what some of their kids sometimes experience. They called it a 'Mito crash'. I've also heard it called a 'Mito storm'. It can last for days at a time and then as quickly and mysteriously as it showed up, can disappear and they recover...usually, that's the hope at least. I had another mother tell me that this kind of thing happened when her mito son was on high doses of steroids. The steroids actually break down the muscles. Both are interesting thoughts, not what I want to hear. There is no cure or treatment. Of course, all this is only coming from mito moms, not a doctor so it could all be way off base but it certainly sounds familiar when I hear these moms talk about their experiences.

He finished his steroids today! Thank goodness! I am praying that we start to see some improvements now that we are done with that evil drug! If this truly is a mito crash...I am going to have to dig deep to find some peace about this. The only other time I have felt this scared was the first time I gave CPR to Shelbie when she was just two years old.

Friday, November 25, 2011

Yesterday, the kids and I watched a documentary called "A Day in the Life". It was pretty random and consisted of just a bunch of random people and how they spend one Saturday in July. It was both intriguing and boring but on Thanksgiving when half the family is sick, what else is there to do right?

Anyways, there was some guy talking about how he has battled cancer with his wife twice and won. He said, "Nothing scares me anymore. I am fearless." Fearless? What would life be like if you were fearless? Does it take a showdown with death at high noon with tumble weed blowing by your rugged boots one dusty Saturday in the middle of a desolate, ghostly town?

I don't know but I can't get that thought out of my head. I have had my own showdown or two with the threats of death or at least crippling disease and I am not fearless. Two days ago, a good friend and her husband were in a head-on collision on the highway. Thankfully, their 10 children were not traveling with them. Both are in intensive care. She broke and/or crushed multiple bones, head contusions, lacerated liver and the list goes on. When she has completed numerous surgeries and months before she can take one little baby step, will she be fearless?

Sometimes, I feel like I have survived the hardest thing ever and nothing would shake my world. Most times, I feel scared. Always hyper alert for danger, trouble, freak accidents. When my kids walk out the door, I sometimes can't get the worst case scenarios out of my mind. I hope the day will come when I will have enough hope, enough faith, to say that I too am fearless. Until then...I guess I will try to enjoy this white knuckle ride.

Tuesday, November 22, 2011

Well, the doctor was in bright and early this morning. He has done a great job with Spencer and I really appreciate all the time he has put in to finding answers.

The good news is that Spencer's brain scan for tumors, lesions or abnormalities in the lining of the nerve sheathing all came back normal! Whew! That leaves the problem with his palate, disappearing gag reflex and throat pain more than likely mitochondrial or possibly autoimmune but the steroids haven't helped at all so that's not as likely. So, we are back to no treatment, no cure. Though it hasn't been confirmed by the Neurologist, it follows the pattern of mito problems.

The stomach and vomiting issues are another perplexing issue altogether. The doctor said what Spencer has is not Gastroenteritis or 'stomach flu'. He said that when his immuno compromised patients have to take high doses of steroids, it overwhelms their system and causes "malignant vomiting" which is what Spencer has been experiencing. So the steroids, combined with the fact he ate 7 slices of jalapeno pizza without his pancreas medication just shredded his entire GI tract. The doctor doesn't think things will resolve until he is done the run of steroids. We still have 8 more days of steroids!! Once we finish those, there will be more testing and more stuff to figure out how to get Spencer back on track with his health. Shelbie has sort of taken the spotlight this year with her chronic immune problems but in the background, Spencer has been suffering just as much but in different ways.

I am bringing Spencer home today and we are armed with pain meds, anti nausea meds and hopefully the vomiting won't come back. He is barely able to eat even a couple bites of toast without getting totally sick.

The doctor reiterated the fact that kids' metabolic system is really not functioning well and whether that is all mitochondrial or some other genetic breakdown, it remains to be seen. He had a big conference with an Internist and Radiologist yesterday and they all feel like bigger things are brewing...but what? So, the mystery continues!

It's so hard to hear that! It's so hard because I have to change my mindset. I tend to wait before taking the kids to the doctor; keep hoping that things will resolve and then we end up in a crisis like we have this week! It stinks. It blows my mind. You would think after 19 years of this, I would be a little further along the learning curve. It's so hard to find a balance between, 'this will pass' and 'it's time for the hospital'.

Now, my instinct is to go home, clorox my house and anyone who darkens my door! I want to quiz anyone who comes around, make sure they aren't dragging germs along with them. Basically, I'm all about keeping them in a bubble. This week has just about done me in! I know that is totally impossible and irrational but setbacks like this really make me feel crazy and out of control. I know it will pass but for now, I'm hyper alert!

Monday, November 21, 2011

I wasn't quite expecting the whirlwind of crazy and insane we are caught up in. I've been keeping an eye on Spencer's throat and palate and that alone has been a cause for worry. The prednisone has taken it's toll on him. One thing I totally forgot about was the fact that prednisone can suppress the immune system drastically.

Yesterday, Spencer started throwing up. It went on every 30 min for nearly 9 hours. Sometime in all that, he developed a really bad pain in his abdomen and a high fever. We brought him in to the ER thinking maybe a little fluid and some IV anti nausea meds were in order. Instead, we ended up with CT scans, chest xrays, morphine, lots of fluid because he was severely dehydrated and blood cultures. This is not your typical gastroenteritis but what is it?

Well, we are sort of dealing with two seperate beasts. The throat/cranial nerve problem and the stomach issue. Part of the stomach problem is that he had 7 slices of pizza covered with jalapenos late Saturday night. He ate all that without taking his pancreatic enzymes. It was just too much fat for his body to manage. Spencer has chemotaxis of the white cells which means, he has plenty of white cells but they don't work very well, they don't move very fast to the source of infection so small, insignificant viruses we all deal with can get quickly out of hand. The prednisone aggrevates his immune system and makes it even worse than it is. We are waiting for blood cultures still.

The second beast is the cranial nerve problem. When our Oncologist came in this morning, he said he didn't want to wait for the prednisone to work or not. He wants to know sooner than later if Spencer has lesions in his brain, as in Multiple Sclerosis or even tumors that are pressing on the cranial nerve. If those tests come back normal, then it pretty much seals the deal for Mitochondrial disease.

Aside from being extremely tired in more ways than I even knew existed, I am not anxious about the results. I think the stomach issues will resolve when the prednisone is out of his system. Whatever the outcome of his neurological problems, we will deal with it as it comes.

So, those are the nuts and bolts of it all. Of course, my thoughts are running much deeper than just the facts. I will sort those out and save for another day.

Friday, November 18, 2011

We got the official letter from Make A Wish on Wednesday and Spencer has passed all the requirements to be granted a wish! The best part was seeing a smile creep across his face as he opened and read the letter. It's been a long while since I saw my boy smile, a real smile.

Now comes the arduous task of deciding on a wish. There is no shortage of ideas and suggestions but I know Spencer and he is a deep thinker and will put alot of thought into the process. He has to choose his top three wishes and then Make a Wish decides which one to grant him.

Think about what you would wish for? It's so hard! I have no idea what I would wish for. It will be interesting to see what he decides. Whatever it is, I just want him to be happy and be able to enjoy it.

The steroids are taking a toll on him and it's hard to watch. We are on the downside now and I was hoping there would be a change in his symptoms and pain but unfortunately, there is no change. Still pain, still no gag reflex. Still worried.

For now, we are going to focus on the fun that Spencer has in store for him!

Wednesday, November 16, 2011

I think I have uttered these words before but it's true, Prednisone sucks! I think it's killing poor Spencer. Not literally but the poor kid. Sheesh!

Tonight his face was super flushed and his body temperature has dropped to only 95.6! His face is getting swollen, his appetite is huge which is a problem! A problem only because all the food I bought over three weeks ago on my last grocery store run was food that is to make things from scratch so there are no 'grab and go' things in house. I felt so bad for him tonight, he ransacked the kitchen trying to find anything he could eat right away. I offered him grilled cheese, pancakes and eggs, nachos, tacos, toast with peanut butter, ice cream, chocolate chip cookies you get the picture. He couldn't wait for me to make something, and it was nearly 11pm. He finally let me make him some toast which I slathered with butter and peanut butter! I know he went to bed starving.

Let's see, what else. He's dizzy and 'whirling', the skin on his fingers is peeling off in sheets, he has a headache and his eyes, muscles and joints hurt.

I'm sure prednisone won't kill him but I'm telling you, it kills me! It kills me to watch him get so sick on something that is suppose to make him better!

As for Shelbie, she is hanging in there with a bad cold. IVIG is still not scheduled and I'm getting frustrated because if she doesn't get it this week then it means she will get it next week and be sick with the side effects for Thanksgiving! Last Thanksgiving she was in the hospital with a platelet count of 4 or some crazy number like that. I just want one holiday this year with everyone healthy and happy but that might be asking for too much. We will be happy, we always make the best of whatever comes but it would be great to be healthy. She's had some new symptoms crop up like a patch of hair is missing from her head, she has a tremor in her hands and her right arm is numb. I have no idea what to make of these symptoms.

Sam...as I say this, I am not just knocking but pounding on wood...he is doing okay. At least nothing unusual just his regular, nightly nausea and stomach cramping.

Sunday, November 13, 2011

I have been sticking pretty close to my online support groups for both Mitochondrial disease and Shwachman Diamond. It's not that they solve any problems for me but there is a comfort knowing that all the flood of feelings I experience from one moment to the next are normal.

One mom today, wrote a message to someone else in the mito group. She verbalized so well what I feel all the time. She talked about how she keeps her life in 'boxes'. The boxes contain emotion too overwhelming to deal with.

I keep my own life compartmentalized. I think it's the only way to manage everything. I guess the perfect example is my blogging. Three separate blogs with completely different content. In addition to all these blogs, I have two or three different journals with even more thoughts and feelings, different from anything read in my online world. It may seem strange but it helps. It keeps the chaos controlled.

When it's time to deal with one part of my fractured life, I can carefully lift the lid and let a little escape at a time rather than balancing all the little parts at once.

This same mito mom who I read about today also suggested that coping with the unknown is a skill. Like anything else, it takes practice and practice makes perfect. I can not visualize the day when I will not be thrown by a new problem, a new diagnosis, a new worry and unknown of living with rare, chronic disease. I have no idea if I will ever see that day but each setback is a chance to practice increasing my faith and starving my fear.

Friday, November 11, 2011

Well, not good news from the Ear, Nose, Throat doctor yesterday. Not good at all. Spencer is not getting a cold, or tonsilitis, nor does he have strep throat. He has a neuropathy. What is a neuropathy? As far as my simple mind understands, it is dying of the nerves. The nerve that the doctor thinks is being damaged is the cranial nerve.

The cranial nerve is responsible for things like the gag reflex. So, the million dollar question...what is causing the nerve damage? He said it is one of two things. One, it could be that there is an autoimmune process taking place that is attacking the nerve. Two, it could be that the defective mitochondria is killing the nerve. Theory one can be treated. Theory two can not.

We are trying the treatment for autoimmune/viral infections of the nerve which is a super, rockstar size dose of prednisone. I hate prednisone! It makes my kids turn into mean, angry people who feel sicker than before they started it. We don't have a choice so the next 25 days are going to be interesting.

If the symptoms of pain and fullness subside, then it will confirm an autoimmune attack of the nerves and we make a plan from an immunology standpoint. If the symptoms are not better, then we get a confirmed Mitochondrial problem for which there is no cure and it is progressive.

Of course, I am praying fiercely that it is just a virus or bacteria and the medication will work. I always know that when we experience these little breaths of calm in our life like we have the last 3 or 4 weeks, it will be followed by a tornado of events that we never saw coming. Kind of crazy.

Spencer refuses to talk to me about how he's feeling, he just grumbles about how much he hates the doctor and the doctor is stupid and the doctor had no idea what he was talking about and, and, and which is a 17 year olds secret language that means...this sucks and I am scared and what if they can't fix me?

Thursday, November 10, 2011

If I delete my last post, take back all I said about things being so great...can I turn back time and the nonsense today will cease to exist? Blech!

Shelbie woke up sick! I guess it makes sense since we are a week and a half from infusion even though it feels like the last one was more than 6 months ago! Stress has a way of making time drag! Last night, my kids had friends over who only finished throwing up 24 hours ago, others who have been around all kinds of sick people at work. No matter how many times I try to tell them stay away from sick people, it never works.

Then there's Spencer. He's been complaining of a sore throat for a few days now. I figured it was a little cold but when I look in his throat, there is absolutely no redness whatsoever, no sign of infection, he doesn't have a fever, achiness or any other symptom of a cold or otherwise. The pain is inside and stabbing and he feels pressure and fulness in his throat. Then a new symptom has developed, a weird one. He has lost his gag reflex. Strange!

Spencer is my kid who gags when he brushes his teeth. Every morning, I can tell when he is brushing his teeth because he is up there coughing and gagging. Last night, he demonstrated how he can stick his finger clear down his throat, past his Uvula, that little dangling thing, wag his finger back and forth and no gagging. Weird.

I jumped on my mito group and sent out an email asking if any other mito kids had experienced anything like it. I did not like the answers that started rolling in just minutes later. Lots! Lots of people have experienced this in the mito world. One lady, who has confirmed mito said that she experience that and it was the dying of some cranial nerves! Another mom reported that her daughter experienced a loss of the gag reflex and it was her salivary glands dying off due to mito. Oh my gosh...I hope beyond hope that this is not a mito problem. I really hope it's just some random infection inside his tonsils that we can treat with a little pill of some sort!

I also called out to our Neurologist in Seattle to see if he can give us some direction and this afternoon, we are off to the Ear, Nose, Throat doc. Here we go...

Monday, November 7, 2011

BUT...things have been pretty quiet around here from a health standpoint. I hate to say it because it seems as soon as you mention how great things are, they turn to not so great very quickly.

Last week, Shelbie stayed pretty healthy, all things considered. I feel like her body is finally accepting the plasma better and all the treatments are starting to pay off. It kind of gives me a false hope that she is 'cured' but I know it's not real. Well, it's real but it's not her body doing any work, just the medicine.

Spencer had a few glitches in his week. He broke his toe playing soccer in gym class, had the pneumonia shot which irked him some and continued with his daily nausea routine but he managed pretty well. He got a job over the weekend so that is great but means he will be gone everyday after school for a few hours and I worry that it will be too much for him but we will see how it goes. He's really excited and I am too.

Sam scared me this week but other than the scare is doing well. He seems to be the healthiest of the bunch even though his bone marrow is just as empty as the others. We have known now for some time that he has the mito defect for Liebers optic neuropathy. Liebers is failure of the optic nerve which results in blindness. All the kids have markers for it but the doctors can't tell us for sure what it means. A few months ago, Sam got a black eye. At the same time, he had a problem with his peripheral vision which I attributed to the black eye and inflammation. Well, this past week, it happened again only in the other eye but it had not been injured or anything. He complained about it for two days. I was trying not to worry and take the wait and see approach but I was worried. I think I asked him every hour, "Is your vision back?" It did resolve but I don't like it when weird stuff like this happens without an explanation.

At any rate, I am glad for the break in trauma and crisis from chronic health problems, it has freed up time to deal with other things falling apart. Sadly, I know the problems will be coming around again.

Thursday, November 3, 2011

Yesterday, I learned a few things. Spencer had to get a Pneumonia vaccine because his most recent immunology tests showed that he had little resistance, in fact hardly any at all, to the Pneumonia bacteria. In my mind, I just thought, Oh well, we will have to be extra careful he doesn't get pneumonia. He had it just a few months ago but still, has not built up a resistance.

While sitting in Short Stay at the hospital yesterday waiting for what was suppose to be a simple vaccine, the nurse handed me some literature on the Pneumovax vaccine. I did not know that the Pneumonia bacteria is actually responsible for causing ear infections, strep throat, meningitis and a host of other problems! Spencer has always been plagued with strep bacterias, ear infections and has had meningitis which turned into encephalitis.

In four weeks, we have to have labs drawn to see if his body has formed any titers to the vaccine. If he hasn't then, the doctor said he will most likely have to start IVIG for the winter months at least to give him a little protection. Blech! Not excited about the prospects of that! I wonder if we could get a family discount, share a room. Anyways, we will just have to wait and see. Just for the record, I don't cross my fingers anymore, that changes nothing!

The other interesting thing I learned is that Red Heads are more sensitive to everything, more than anyone else with a different color of hair. Have you ever heard that? Before the nurse gave Spencer the shot, she said, "Okay mom, you know what they say about red heads..." In my mind, I'm thinking, they have a fiery temper? What does that have to do with vaccines? That can't be right so I said, "No, what do they say about red heads?"
"They are far more sensitive to things happening in their body so keep a very close watch on him tonight and over the next few days. He could get some bad reactions, worse than most people."
I had no idea. Looking back, I think she may be right.

This morning, the pain in Spencer's arm is so bad he can hardly lift it! Coincidence? I don't know, it's another pain, another dose of ibuprofen, another day for us, red hair or not!

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"For a small moment have I forsaken thee, but with great mercies will I gather thee...I hid my face from thee for a moment, but with everlasting kindness will I have mercy on thee, saith the Lord thy Redeemer...For the mountains will depart and the hills shall be removed, but my kindness shall not depart from thee, neither shall the covenant of my peace be removed, saith the Lord that hath mercy on thee.

Oh thou afflicted, tossed with tempest, and not comforted! Behold, I will lay thy stones with fair colors, and lay thy foundations with sapphires. And I will make thy windows of agates and thy gates of carbuncles, and all thy borders of pleasant stones...great shall be the peace of thy children. Thou shalt be far from oppression for thou shalt not fear, and from terror for it shall not come near thee." 3 Nephi 22:7-14